A 10-year-old girl with uncorrected tetralogy of Fallot with pulmonary atresia presented with fevers of unknown origin and left lung infiltrates. At autopsy, necrotizing vascular changes resembling those of severe pulmonary hypertension (grade VI in the Heath-Edwards classification) were confined to the left lung. Pulmonary blood flow and pressure were greater in the left lung and were provided by an enlarged collateral artery arising directly from the descending thoracic aorta. To our knowledge, this is the first report of necrotizing arteritis of the pulmonary arteries in uncorrected tetralogy of Fallot with pulmonary atresia.
Tetralogy of Fallot Pulmonary hypertension Necrotizing arteritis Pulmonary atresia with ventricular septal defect
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Haworth S (1980) Collateral arteries in pulmonary atresia with ventricular septal defect: A precarious blood supply.Br Heart J 44: 5–13PubMedGoogle Scholar
Haworth S, Macartney FJ (1980) Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.Br Heart J 44: 14–24PubMedGoogle Scholar
Jefferson K, Rees S, Somerville J (1972) Systemic arterial supply to the lungs in pulmonary atresia and its relation to pulmonary artery development.Br Heart J 34: 418–427PubMedGoogle Scholar
Johnson RJ, Sauer U, Buhlmeyer K, Haworth SG (1985) Hypoplasia of the intrapulmonary arteries in children with right ventricular outflow tract obstruction, ventricular septal defect and major aortopulmonary collateral arteries.Pediatr Cardiol 6: 137–143PubMedGoogle Scholar
Macartney F, Deverall P, Scott O (1973) Hemodynamic characteristics of systemic arterial blood supply to the lungs.Br Heart J 35: 28–37PubMedGoogle Scholar
Macartney FJ, Scott O, Deverall PB (1974) Hemodynamic and anatomical characteristics of pulmonary blood supply in pulmonary atresia with ventricular septal defect—including a case of persistent fifth aortic arch.Br Heart J 36: 1049–1060PubMedGoogle Scholar
McGoon MD, Fulton RE, Davis GD, et al (1977) Systemic collateral and pulmonary artery stenosis in patients with congenital pulmonary valve atresia and ventricular septal defect.Circulation 56: 473–479PubMedGoogle Scholar
Rabinovitch M, Herrera-deLeon V, Castaneda AR, et al (1981) Growth and development of the pulmonary vascular bed in patients with tetralogy of Fallot with or without pulmonary atresia.Circulation 64: 1234–1249PubMedGoogle Scholar
Wagenvoort CA, Wagenvoort N (1977)Pathology of pulmonary Hypertension. John Wiley & Sons, New York, pp 56–95, 311–323Google Scholar